[CIS PIDD] [cis-pidd] selective IgM deficiency

Mon May 15 12:35:19 EDT 2017

Hello
Thanks to you all for your comments.Here are the answers to your questions and a new information that I did´t tell you ( eosinophilic colitis and eosinophilia)...see later 
1.    Question---: Its not vasculitis? whats the lungpathologic analysis?: 

    Answer:  

o  Bronchoneumonia with focal signs oforganization

o  Bronchestasis

o  Multifocal bronchiolitis

o  Hiliar lymphonodes with reactivehiperplasia and mild to moderate antracosis

2.   Question: Ishe aspirating? 

         Answer: A study to confirm he isaspirating has not been done jet

3.  Question:Complement deficiency considered?

         Answer: We didn´t consider thatoption, we only have a mild low C3, C4 is normal. CH50 has not been measuredand we do not have acceso to AH50

4.  Question:H. influenzae isolated with sputum or BAL with EVERY episode? 

         Answer: No, only in one. In the othersthe celularity was of neutrophils predominance as well, but cultures werenegative

5.  Question:IN other words, is this absolutely without a doubt secondary to bacterialinfection or could it be inflammatory pulmonary disease?

Answer:I am not absolutely sure that this are bacterial infections and could be an inflammatorypulmonary disease

IThink that H. Influenza could be a colonization but it was consistent with celulariryin BAL and with leucocitosis and neutrophilia in blood 

6.  Question:Could it be (functional?) hyposplenism? 

Ithas been reported to be associated with low IgM memory B cells and sometimeseven with low serum IgMs. 

Hemophilusis compatible (even though no sepsis?).

Answer:memory B cell studies are under way ( I don´t have the results yet).

Thepatient will have an upper endoscopy and colonoscopy this week to look for:seronegative celiac disease, GER and because a new information was recovered:

Hehas endoscopies from 2015 with the following results:

“Mildnon specific colitis with stromal edema and focal increase of eosinophils (maximal count 55 per camp ). Ileon histology is normal

Looking for blood eosinophils (we have access from 2016 until now) he has have eosinophilic counts from 590 up to 1890 ( percentages from 4.7 to 19%). Total IgE is normal 
I would be pleased to read more comments from you
Patricia  

    El Viernes 12 de Mayo de 2017 2:14, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> escribió:

Hi    Have you considered gastroesophageal reflux?  (the bypass surgery 2012)    In the literature selective IgM deficiency has rather mild phenotype, mainly allergy.    Best regards    Timi Martelius Infectious Diseases Helsinki University Hospital    Lähettäjä: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net]Puolesta CIS-PIDD
Lähetetty: 11. toukokuuta 2017 22:51
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: [cis-pidd] selective IgM deficiency    Dear colleagues,    I am Patricia Roessler, a chilean Clinical Immunologist. . I would like your opinion about a clinical case, if it is possible.   The patient is a male of 50 years, previously healthy until 2015 when he had a mild influenza H1N1 infection. Then on, he had have 6 episodes of pneumonia without fever but with leucocytosis, neutrophilia and elevated ESR and CRP. He was treated with oral antibiotics with regular response so he needed intravenous antibiotics to recover from this pneumonias. The infections were always in the same lobe ( inferior right lobe). He had a Fibrobronchoscopy with BAL in November 2016. There was hypercellularity with 95% of neutrophils and Haemphylus Influenzae grew in cultures. The biopsy was inespecific. In Lung Images no anatomic defect could be detected but as the pneumonias were always in the same place a lobectomy was done in March 2017. In Abril 2017 he had a pneumonia, now in the left lung, with fever and similar characteristics in general labs than previous episodes.   His infectious history was anodyne, except for recurrent tonsillitis in infancy ( he went a tonsillectomy). He refers no sinus infections, although in a a PET CT done in this hospitalization a sinusitis was described.   He had a gastric by-pass surgery because of obesity and diabetes in 2012 ( both of this conditions reverted until now)   He has frecuent diarrheas last year.   In Immunologic studies he has:   November 2016:  IgG 1233  IgA 436 (mild elevation) IgM 31 (low)    Normal IgG subclasses   80% of anti pneumococcal antibodies were in protective titles (never vaccinated)    April 2017:  IgG 919 IgM 22 IgA 327    Lymphocytes subpopulations were all normal.   As an IgM deficiency was found he went an hematologic evaluation with serum electrophoresis and immunofixation being normal, and a PET CT without signs of an occult neoplasm. The patient denied a Bone Marrow biopsy.   A malabsortion syndrome was diagnosed (low carotene, very low D vitamins and zinc levels, normal albumin ) and assumed to be secondary to his by pass.  Celiac disease serology is negative    I think that this patient has a primary selective IgM deficiency but I don´t know well how to treat him. - Do you think he needs more immunologic studies ( B memory cells and anti Haemophylus influenza antibodies are in course, antibodies to protein antigens have not been measured).   - Do you think he can benefit from IVIG? (I have read that patients with selective IgM deficiency and low anti pneumococcal antibodies can have a good responde but is not the case of this patient)   - Do you think that Pentaglobin could help (I have no experience with this IVIG)?   Meanwhile he is receiving conjugated pneumococcal an H. Influenza vaccines and I have put him in prophylactic antibiotics ( daily sulphametoxazol/thrimetropin)      Thank you very much and I hope you can help me   Best regards,    Patricia  --- You are currently subscribed to cis-pidd as: timi.martelius at hus.fi. To unsubscribe click here: http://cts.dundee.net/u?id=96396923.f74241bc90eb204d900c577ce46ce57c&n=T&l=cis-pidd&o=4369783 (It may be necessary to cut and paste the above URL if the line is broken) or send a blank email to leave-4369783-96396923.f74241bc90eb204d900c577ce46ce57c at lyris.dundee.net  ---  You are currently subscribed to cis-pidd as: patar20000 at yahoo.es.  To unsubscribe click here: http://cts.dundee.net/u?id=96396837.2f6bf9c2fc4064133107480c2594d9af&n=T&l=cis-pidd&o=4370526  (It may be necessary to cut and paste the above URL if the line is broken)  or send a blank email to leave-4370526-96396837.2f6bf9c2fc4064133107480c2594d9af at lyris.dundee.net

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